Associated Disorders of Chiari Type I Malformations

Abstract

There exists a plethora of diseases affiliated with Chiari malformation type I (CM I) and certainly more to be discovered in the future. While the final outcome of CM I may be the same, the strength of the correlation and pathophysiological mechanisms of each differs greatly, and some may be spurious associations. Thus, the need for additional genetic research and investigation of CM I continues. This chapter will discuss the conditions associated with CM I in order to potentially shed light on how the pathophysiological mechanism of one condition, no matter how remote, might lead to the development of CM I. It should be noted that many of these associations may be incidental with an asymptomatic hindbrain hernia being identified due to testing for other pathologic entities, for example, endocrinopathies.